Cognitive and speech-language performance in children with ataxia telangiectasia

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Title:	Cognitive and speech-language performance in children with ataxia telangiectasia
Author(s):	Vinck, A. ; Verhagen, M.M.M. ; Gerven, M.; Groot, I.J.M. de ; Weemaes, C.M.R. ; Maassen, B.A.M. ; Willemsen, M.A.A.P.
Publication year:	2011
Source:	Developmental Neurorehabilitation, vol. 14, iss. 5, (2011), pp. 315-322
ISSN:	1751-8423
DOI:	https://doi.org/10.3109/17518423.2011.603368
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/97728
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Subject:	DCN 2: Functional Neurogenomics N4i 4: Auto-immunity, transplantation and immunotherapy NCEBP 10: Human Movement & Fatigue DCN 1: Perception and Action NCEBP 8: Psychological determinants of chronic illness NCEBP 8: Psychological determinants of chronic illness DCN 1: Perception and Action NCEBP 10: Human Movement & Fatigue DCN 1: DCN 1: Perception and Action
Organization:	Medical Psychology Neurology Rehabilitation Paediatrics - OUD tm 2017
Journal title:	Developmental Neurorehabilitation
Volume:	vol. 14
Issue:	iss. 5
Page start:	p. 315
Page end:	p. 322
Abstract:	OBJECTIVE: To describe cognitive and speech-language functioning of patients with ataxia-telangiectasia (A-T) in relation to their deteriorating (oculo)motor function. DESIGN: Observational case series. METHODS: Cognitive functioning, language, speech and oral-motor functioning were examined in eight individuals with A-T (six boys, two girls), taking into account the confounding effects of motor functioning on test performance. RESULTS: All patients, except the youngest one, suffered from mild-to-moderate/severe intellectual impairment. Compared to developmental age, patients showed cognitive deficits in attention, (non)verbal memory and verbal fluency. Furthermore, dysarthria and weak oral-motor performance was found. Language was one of the patients' assets. CONCLUSION: In contrast to the severe deterioration of motor functioning in A-T, cognitive and language functioning appeared to level off with a typical profile of neuropsychological strengths and weaknesses. Based on our experiences with A-T, suggestions are made to determine a valid assessment of the cognitive and speech-language manifestations.